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Hunting for a Diagnosis
WD – 14 / 15
1
Introduction.
 Mr. H, 94 year old farmer from Thalgampala.
 Father of eight children.
 Never had Diabetes, Hypertension and Asthma in the
past.
 No history of prolonged previous hospital admissions.
 Non smoker.
 Has consumed alcohol occasionally in the past.
2
Reason for admission,
 Painful blackening of bilateral finger tips.
 Onset – insidious
 Progressive over a three weeks.
 Involved bilateral 2nd, 3rd, 5th finger tips on arrival.
 bilateral toes not involved.
 No history suggestive of Raynaud's phenomenon
 No history of rashes elsewhere.
3
Review of systems
 No history of intermittent claudication
 No history of fever at any point of illness
 No Cough, shortness of breath
 No haematuria
 No history of abdominal pain, especially after meals
 There was no fatigue, recurrent epistaxis, sinus pain,
hearing loss, and arthralgias.
 No history of longstanding joint pain, morning stiffness,
or joint deformities
 No history of snake bite
4
Contd,
 No history of previous blood transfusions.
 No history suggestive MI, TIA in the past.
 No recent weight loss, good appetite.
 No history of prolonged consumption of
medications
5
Examination Findings
 Bilateral symmetrical peripheral gangrene involving
finger tips, feet spared.
 Peripheral pulses - palpable
 BP – 130/80 mmHg in both arms.
 No murmurs were audible.
 No rashes
 No pallor, icterus, lymphadenopathy or organomegaly.
 No abdominal masses.
 No joint deformities or nodules.
 No peripheral or CNS involvement.`
 Fundus : no retinal infarction or thrombosis.
6
7
8
• Problem list:
Problem list:
 Bilateral symmetrical peripheral gangrene
involving finger tips, sparing feet at extreme of
age in a previously healthy man.
10
• Differential diagnosis?
11
Differential diagnosis:
Possibilities include,
1. Vasculitis
2. Recurrent small thromboembolic disease
3. Infective endocarditis
4. Manifestation of a Paraneoplastic syndrome
5. Hyperviscosity syndrome
12
 Investigations:
13
Inflammatory markers
 ESR – 110 mm 1st hour.
 CRP – 30 mg/dl
14
 Urine ward test for albumin – negative
 UFR – No RBC or casts.
15
FBC
Hb – 11.4 mg/dl
WBC – 8400 Cumm3
RBC – 3.37 x 105
Plt - 157000 Cumm3
16
 Blood Picture
 Normocytic normochromic anaemia
 WBC – no left shift or toxic granules
 Platelet within normal range.
 No fragmented RBC
17
 Direct and indirect Coomb’s test
 negative
18
 Blood cultures were repeatedly normal.
19
 Rheumatoid factor – positive
 ANA - negative
20
 VDRL – Negative.
21
 Mantoux test
 negative
22
 Cryoglobulin assay
 Not detected after 72 hrs.
23
 Hep. B surface antigen – negative
 Hep. C antibody - negative
24
 Coagulation profile
 Normal.
25
 Urine BJP – negative.
 Skeletal survey – no lytic lesions detected.
 Serum protein electrophoresis – no
monoclonal band observed.
26
 C-ANCA - negative
 P-ANCA - negative
27
 Complement assay.
 C3, C4 levels - normal
28
 2D – Echo – No vegetations, repeatedly.
29
 USS – Abdomen
 No Para-aortic lymadenopathy
 No hepatosplenomegaly
30
 Duplex scan of bilateral lower limbs.
 No evidence of DVT
31
 Contrast CT abdomen, pelvis and Thorax.
 No abnormality detected
32
How we managed him,
 Multidisciplinary approach
 Rheumatology dept.
 Radiology dept.
 Blood bank
 Vascular surgical team
33
 Desperate measure,
34
 Biopsy
 Gangrene following vasculitis is the favored
microscopic diagnosis.
 No granuloma formation observed.
35
 This scenario fits the ever-widening etiological
spectrum of SPG.
36
 Symmetrical peripheral gangrene (SPG)
 rare clinical syndrome
 characterized by bilateral distal ischemic damage leading to
gangrene in the absence of major vascular occlusive
disease
 Peripheral pulses are usually palpable as a result of sparing
of larger vessels
 The mechanism of vascular occlusion is poorly understood
 A wide array of infective and non infective etiological factors
 Could be associated with sepsis, low-flow states, vasospastic
conditions, myeloproliferative disorders, and hyperviscosity
syndromes
37
38
Thank you!
39

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Hunting for a diagnosis

  • 1. Hunting for a Diagnosis WD – 14 / 15 1
  • 2. Introduction.  Mr. H, 94 year old farmer from Thalgampala.  Father of eight children.  Never had Diabetes, Hypertension and Asthma in the past.  No history of prolonged previous hospital admissions.  Non smoker.  Has consumed alcohol occasionally in the past. 2
  • 3. Reason for admission,  Painful blackening of bilateral finger tips.  Onset – insidious  Progressive over a three weeks.  Involved bilateral 2nd, 3rd, 5th finger tips on arrival.  bilateral toes not involved.  No history suggestive of Raynaud's phenomenon  No history of rashes elsewhere. 3
  • 4. Review of systems  No history of intermittent claudication  No history of fever at any point of illness  No Cough, shortness of breath  No haematuria  No history of abdominal pain, especially after meals  There was no fatigue, recurrent epistaxis, sinus pain, hearing loss, and arthralgias.  No history of longstanding joint pain, morning stiffness, or joint deformities  No history of snake bite 4
  • 5. Contd,  No history of previous blood transfusions.  No history suggestive MI, TIA in the past.  No recent weight loss, good appetite.  No history of prolonged consumption of medications 5
  • 6. Examination Findings  Bilateral symmetrical peripheral gangrene involving finger tips, feet spared.  Peripheral pulses - palpable  BP – 130/80 mmHg in both arms.  No murmurs were audible.  No rashes  No pallor, icterus, lymphadenopathy or organomegaly.  No abdominal masses.  No joint deformities or nodules.  No peripheral or CNS involvement.`  Fundus : no retinal infarction or thrombosis. 6
  • 7. 7
  • 8. 8
  • 10. Problem list:  Bilateral symmetrical peripheral gangrene involving finger tips, sparing feet at extreme of age in a previously healthy man. 10
  • 12. Differential diagnosis: Possibilities include, 1. Vasculitis 2. Recurrent small thromboembolic disease 3. Infective endocarditis 4. Manifestation of a Paraneoplastic syndrome 5. Hyperviscosity syndrome 12
  • 14. Inflammatory markers  ESR – 110 mm 1st hour.  CRP – 30 mg/dl 14
  • 15.  Urine ward test for albumin – negative  UFR – No RBC or casts. 15
  • 16. FBC Hb – 11.4 mg/dl WBC – 8400 Cumm3 RBC – 3.37 x 105 Plt - 157000 Cumm3 16
  • 17.  Blood Picture  Normocytic normochromic anaemia  WBC – no left shift or toxic granules  Platelet within normal range.  No fragmented RBC 17
  • 18.  Direct and indirect Coomb’s test  negative 18
  • 19.  Blood cultures were repeatedly normal. 19
  • 20.  Rheumatoid factor – positive  ANA - negative 20
  • 21.  VDRL – Negative. 21
  • 22.  Mantoux test  negative 22
  • 23.  Cryoglobulin assay  Not detected after 72 hrs. 23
  • 24.  Hep. B surface antigen – negative  Hep. C antibody - negative 24
  • 26.  Urine BJP – negative.  Skeletal survey – no lytic lesions detected.  Serum protein electrophoresis – no monoclonal band observed. 26
  • 27.  C-ANCA - negative  P-ANCA - negative 27
  • 28.  Complement assay.  C3, C4 levels - normal 28
  • 29.  2D – Echo – No vegetations, repeatedly. 29
  • 30.  USS – Abdomen  No Para-aortic lymadenopathy  No hepatosplenomegaly 30
  • 31.  Duplex scan of bilateral lower limbs.  No evidence of DVT 31
  • 32.  Contrast CT abdomen, pelvis and Thorax.  No abnormality detected 32
  • 33. How we managed him,  Multidisciplinary approach  Rheumatology dept.  Radiology dept.  Blood bank  Vascular surgical team 33
  • 35.  Biopsy  Gangrene following vasculitis is the favored microscopic diagnosis.  No granuloma formation observed. 35
  • 36.  This scenario fits the ever-widening etiological spectrum of SPG. 36
  • 37.  Symmetrical peripheral gangrene (SPG)  rare clinical syndrome  characterized by bilateral distal ischemic damage leading to gangrene in the absence of major vascular occlusive disease  Peripheral pulses are usually palpable as a result of sparing of larger vessels  The mechanism of vascular occlusion is poorly understood  A wide array of infective and non infective etiological factors  Could be associated with sepsis, low-flow states, vasospastic conditions, myeloproliferative disorders, and hyperviscosity syndromes 37
  • 38. 38

Notes de l'éditeur

  1. though disseminated intravascular coagulation (DIC) has been implicated as the final common pathway in its pathogenesis.